Abstract
IntroductionExtranodal, natural-killer/T-cell lymphoma of nasal type is a rare but aggressive disease usually presenting as progressive necrotic lesions in the nasal cavity that responds poorly to chemotherapy. In this paper, we report a relapsing, chemorefractory case of extranodal natural-killer/T-cell lymphoma responding to checkpoint inhibitor immunotherapy with pembrolizumab.Case presentationA 32-year-old Hispanic woman with a history of recurrent sinusitis and preseptal abscess presented with a hoarse voice, swelling around the right eye, and diplopia. Laryngoscopy showed infiltrating tissue extending to bilateral laryngeal ventricles and false vocal cords. On immunohistochemical examination of laryngeal biopsy, the neoplastic cells showed positivity for CD3 (cytoplasmic), CD7, CD56, granzyme B, CD30, and Epstein–Barr virus-encoded ribonucleic acid (RNA). Extranodal natural-killer/T-cell lymphoma, nasal type, was confirmed. In the absence of distant organ involvement, the decision was to perform chemotherapy with etoposide, ifosfamide, mesna, cisplatin, and dexamethasone (VIPD protocol) followed by concurrent chemoradiation with weekly doses of cisplatin and two cycles of VIPD as adjuvant treatment. However, 1 month after completion of the treatment; disease recurrence was demonstrated. The patient was scheduled to receive salvage chemotherapy with steroid, methotrexate, ifosfamide, L- asparaginase, and etoposide (SMILE) protocol and CD30-targeting monoclonal antibodies. However, the mass was chemorefractory without response to either l-asparaginase-based salvage chemotherapy in combination with high-dose methotrexate or brentuximab vedotin. However, this case of chemorefractory extranodal natural-killer/T-cell lymphoma, nasal type, responded well to the novel drug pembrolizumab, which was able to control the disease.ConclusionCheckpoint inhibitors are potential treatment option in selected chemorefractory extranodal natural-killer/T-cell lymphoma, nasal type, cases.
Highlights
Extranodal, natural-killer/T-cell lymphoma of nasal type is a rare but aggressive disease usually presenting as progressive necrotic lesions in the nasal cavity that responds poorly to chemotherapy
Case presentation A 32-year-old Hispanic woman, G1P1L1, stay-at-home mom, with a history of recurrent sinusitis and preseptal abscess for 2 years presented with a hoarse voice, swelling around the right eye, and diplopia in July 2018
Since the lymphoma was localized to the laryngeal area with no systemic involvement, the patient received one cycle of chemotherapy in February 2019 with VIPD protocol containing etoposide (100 mg/m2, intravenous infusion, days 1–3), ifosfamide (1200 mg/m2, intravenous infusion, once a day, days 2–4), mesna (330 mg/m2 of mesna was given before ifosfamide, and 480 mg/m2 was given 4 and 8 hours after infusion for 3 subsequent days, intravenous infusion, three times a day), cisplatin (33 mg/m2, intravenous infusion, weekly), and dexamethasone (40 mg, once daily, days 1–4, with G-CSF support)
Summary
We presented a case of a 32-year-old woman with a history of recurrent sinusitis and preseptal abscess who presented with pansinusitis. Unlike salvage chemotherapy and CD30-targeted immunotherapy with grade 3 toxicities, immunotherapy with pembrolizumab was tolerated well with minimal grade 1–2 toxicity. This case suggests checkpoint inhibitors as a potential treatment option in selected chemorefractory ENKTCL-NT cases. Patient perspective The patient was very happy with being switched from high-dose chemotherapy that needed hospitalization to outpatient immunotherapy. She has tolerated immunotherapy with minimal side effects, mostly mild fatigue. She has been very happy with the results, including the overall disease control as well as resolution of the eye swelling and the change of voice back to normal
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